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Anti-Dysferlin (N-terminal region) antibody produced in rabbit

产品编号:3358878
规格:~1.5 mg/mL, affinity isolated antibody
包装规格:200 μL
产品类别:进口试剂
品牌:Sigma-Aldrich
优惠价:立即咨询
产品价格
产品编号包装单位单价(元)国内现货国外库存询价单
3358878200 μL5040
产品别名

Anti-Dysferlin (N-terminal region) antibody produced in rabbit

Anti-Limb girdle muscular dystrophy 2B

Anti-DYSF

基本信息
NACRES
NA.41
General description【一般描述】
Dysferlin is a transmembrane protein, that belongs to the ferlin-1 family of proteins including myoferlin and otoferlin. It is homologous to the C. elegans fer-1 protein. Dysferlin is expressed in kidney cells, skeletal and cardiac muscle.
Dysferlin is encoded by the gene mapped to human chromosome 2p13.2
Specificity【特异性】
Anti-Dysferlin (N-terminal region) specifically recognizes human and mouse Dysferlin.
Immunogen【免疫原】
synthetic peptide corresponding to a sequence in the N-terminal region of human dysferlin (GeneID: 8291), conjugated to KLH.
Application【应用】
Anti-Dysferlin (N-terminal region) antibody produced in rabbit has been used in:
  • immunoblotting
  • immunoprecipitation
  • immunofluorescence

Biochem/physiol Actions【生化/生理作用】
Dysferlin is implicated in membrane fusion events. It plays a role in membrane repair processes, such as the ability to reseal the sarcolemma upon muscle injury. Dysferlin localization in the membrane and trafficking is impaired by mutations in caveolin-1 and -3. Mutations in the dysferlin gene are associated with limb-girdle muscle dystrophy type 2B (LGMD2B), distal anterior compartment myopathy and related Miyoshi myopathy.
Physical form【外形】
solution in 0.01 M phos­phate buffered saline, pH 7.4, containing 15 mM sodium azide.
产品性质
biological source【生物来源】
rabbit
Quality Level【质量水平】
200
conjugate【偶联物】
unconjugated
antibody form【抗体形式】
affinity isolated antibody
antibody product type
primary antibodies
clone【克隆】
polyclonal
form【形式】
buffered aqueous solution
mol wt【分子量】
~250 kDa
species reactivity
human, mouse
enhanced validation【增强验证】
recombinant expression
Learn more about Antibody Enhanced Validation
concentration【浓度】
~1.5 mg/mL
technique(s)
immunocytochemistry: 7-14 μg/mL using differentiated C2C12 myoblasts
immunoprecipitation (IP): 5-10 μg using HEK-293T cells over-expressing human dysferlin
western blot: 0.5-1.0 μg/mL using HEK-293T cells over-expressing human dysferlin
UniProt accession no.【UniProt登记号】
O75923
shipped in【运输】
dry ice
storage temp.【储存温度】
−20℃
Gene Information
human ... DYSF(8291)
mouse ... Dysf(26903)
产品说明
Storage and Stability【储存及稳定性】
For continuous use, store at 2-8 ℃ for up to one month. For extended storage, freeze in working aliquots. Repeated freezing and thawing, or storage in “frost-free” freezers,is not recommended. If slight turbidity occurs upon prolonged storage, clarify the solution by centrifugation before use. Working dilutions should be discarded if not used within 12 hours.
Disclaimer【免责声明】
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
安全信息
Storage Class Code【储存分类代码】
12 - Non Combustible Liquids
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